The Diseases

PemphigusPemphigus is a group of organ specific autoimmune diseases characterized by the production of autoantibodies to desmogleins. They are mucocutaneous blistering diseases that demonstrate a loss of cohesion of the epidermal cells (acantholysis) which result in the formation of clefts in the epidermis. Autoantibodies directed against adhesion molecules cause epidermal keratinocytes to separate, resulting in intraepidermal bullae. There are two histological types of pemphigus: deep (e.g., pemphigus vulgaris) and superficial (e.g., pemphigus foliaceus). They differ in the epidermal layers that are affected, in the clinical manifestations of the diseases, and in the associated immunologic abnormalities. Pemphigus has an incidence of 1-10 new cases per 1 million people worldwide, with variations in different regions and among different ethnic groups. Incidence is increased in patients of Ashkenazi Jewish descent and those of Mediterranean origin as high as 16 to 32 cases per million people. and pemphigoidPemphigoid is a group of subepidermal, blistering autoimmune diseases that primarily affect the skin, especially the lower abdomen, groin, and flexor surfaces of the extremities. Here, autoantibodies (anti-BPA-2 and anti-BPA-1) are directed against the basal layer of the epidermis and mucosa. The condition tends to persist for months or years with periods of exacerbation and remission. Localized variants of the condition have been reported, most often limited to the lower extremities and usually affecting women. There are two predominant types of pemphigoid: mucous membrane pemphigoid (MMP) also called cicatricial pemphigoid, and bullous pemphigoid (BP). Pathogenesis and management are quite different for these conditions. Scar formation in mucous membrane pemphigoid can lead to major disability. are rare, autoimmuneIn an autoimmune disease, the body's immune system attacks its own organs or tissues by producing autoantibodiesantibodies against the self (antibodies against the self). diseases that affect a very small percentage of the population. There are many different autoimmune diseases, and they can each affect the body in different ways. Pemphigus vulgaris, the most common of the pemphigus diseases, affects the skin and mucous membranes. Ultimately, damage to certain tissues by the immune systemA properly functioning immune system produces antibodies. Like little soldiers, they attack hostile, foreign viruses and bacteria. In a person with P/P, however, the immune system erroneously perceives as foreign one or more proteins that naturally occur in the skin and/or mucous membranes. The immune system responds by producing autoantibodies that attack these proteins. may be permanent.

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Pemphigus and pemphigoid are not contagious. They cannot be transmitted by person-to-person contact or by exchange of blood products or other bodily fluids. Two people in the same family are rarely affected by it. In fact, no autoimmune disease has ever been shown to be contagious or “catching.”